Interdisciplinary treatment of the patient with adenoid cystic carcinoma of the Bartholin’s gland resulting in 15 years’ survival: a and review of literature

Abstract

Carcinoma of the Bartholin's gland is very rare, comprises below 2% of Bartholin's gland lesions and adenoid cystic carcinoma (ADC) is one of the most uncommon variants and accounts for 10-15% of Bartholin's gland malignancies. There is no consensus on treatment of ADC of the Bartholin's gland: reported cases were treated with local excision or vulvectomy with or without lymphadenectomy followed or not by radiotherapy. The survival of patients varies significantly, so we present a case of interdisciplinary treatment of ADC resulting in 15 years’ survival. The patient was initially treated with local excision, but the margins were not clear. Then vulvectomy, inguinal lymphadenectomy and adjuvant brachytherapy were performed resulting in 7 years free of the disease. Relapses were excised by abdominoperineal amputation of the rectum and distal part of the vagina with sigmoideostomy, excisions of local recurrences in vagina and metastasectomy of isolated lung metastases. The patient died manifesting multiple lung metastases 15 years after the initial diagnosis. Based on our experience and world literature, in cases of adenoid cystic carcinoma of the Bartholin's gland, vulvectomy with or without lymphadenectomy should be considered as a treatment of choice and in patients with positive margin, surgery should be extended by adjuvant radiotherapy.

Adenoid Cystic Carcinoma of Vulva-11 Years’ Single-Institution Experience

“…Nowak et al [10] also highlight the need for radical excision procedures for this disease. Quality of life issues need to be considered without compromising treatment.…”

29-year-old with dyspareunia and vulvar mass: An unusual diagnosis of Bartholin's gland carcinoma

“…Most cases of adenoid cystic carcinoma of the Bartholin's gland present in seemingly benign ways. The most common complaints are burning pain, palpable mass, and swelling ( Akbarzadeh-Jahromi et al, 2014 ; Alsan et al, 2011 ; Bhalwal et al, 2016 ; Chamlian and Taylor, 1972 ; Hsu et al, 2013 ; Hwang et al, 2012 ; Nowak et al, 2014 ).The burning sensation is thought to be secondary to perineural involvement. Initial misdiagnosis or delayed diagnosis may occur in up to 50% of patients ( Chamlian and Taylor, 1972 ) due to the overlap of these complaints with benign conditions, such as cyst or abscess ( Chamlian and Taylor, 1972 ; Hsu et al, 2013 ) or endometriosis ( Akbarzadeh-Jahromi et al, 2014 ).…”

A Case of Adenoid Cystic Carcinoma Mimicking a Bartholin Cyst and Literature Review

“…Literature review supports wide local excision, with or without inguinal lymphadenectomy [ 13 , 14 ]. Post-op adjuvant radiotherapy is generally recommended when there is a positive surgical margin, deep local and perineural invasion, and local recurrence [ 15 , 16 ]. Data is lacking for the role of chemotherapy and biological treatment of BG-ACC.…”